Primary Abdominal Lymphangioleiomyomatosis with Mucosa-Associated Lymphoid Tissue Lymphoma
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Primary Abdominal Lymphangioleiomyomatosis with Mucosa-Associated Lymphoid Tissue Lymphoma Bo Song 1,2 & Jia-xin Yan 3 & Ji-chun Zhao 1 Received: 16 March 2020 / Accepted: 23 March 2020 # 2020 The Society for Surgery of the Alimentary Tract
Keywords Abdominal lymphangioleiomyomatosis . Mucosa-associated lymphoid tissue lymphoma . Case report . Diagnosis;treatment
Case Presentation A 25-year-old man was admitted to our hospital with a 1-year history of intermittent right upper abdominal pain. He denied any other abdominal symptoms, including early satiety, flatulence, gastrointestinal bleeding, or vomiting throughout the past 1 year; he had no history of cough, expectoration, asthma, fever, or dyspnea, and other respiratory symptoms. There was no significant family history, particularly of tuberous sclerosis. All physical examinations were normal. He was diagnosed with hepatitis B 8 years ago. All of his laboratory test results, including routine blood examination, liver and kidney function, tumor markers, urine, and stool examinations, were all normal. On abdominal ultrasonography (US) which was performed in another hospital, a right abdominal mass measures 15 × 8 × 8cm3 with homogeneous segmentation was noted. An abdominal contrast-enhanced magnetic resonance imaging (MRI) revealed a massive cystic signal mass comprised of multiple small cysts, which were about 17 × 16 × 7 cm3 in dimension with imperceptible-wall or thin-wall with no enhancement, diffusely distributed or scattered in intraperitoneal
* Ji-chun Zhao [email protected] 1
Department of Vascular Surgery, West China Hospital, Sichuan University, Chengdu, China
2
Department of Gastrointestinal Surgery, Sichuan Cancer Hospital & Institute, Sichuan Cancer Center, School of Medicine, University of Electronic Science and Technology of China, Chengdu, China
3
Department of Pathology, Sichuan Cancer Hospital & Institute, Sichuan Cancer Center, School of Medicine, University of Electronic Science and Technology of China, Chengdu, China
mesenteric region of right upper quadrant (Fig. 1). In DWI images, the signals had been changed from high to moderate then to low as the b value increased from 0 to 500, then to 1000; this highly represented protein-containing inside the lesion. Meanwhile, slightly thicker wall in the distal segment of ileum was observed, which was confirmed with the diagnosis of lymphoma later by pathology. His chest X-ray showed no abnormality. Under the suspicious diagnosis of uncommon abdominal space-occupying lesion, to get a clear diagnosis and treatment, an open abdominal exploration surgery was performed. During the operation, a yellowish sponge-like mass with about 15 cm in diameter located in ascending colic mesenteric region was found (Fig. 2). On gross observation, the mass was soft, segmental, and without obvious capsule. It was filled with clear jelly-like inner-materials. The major part of the mass was attached closely to the anterior wall of duodenum and inferior vena cava. No metastatic lesions were detected in ot
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